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Fibrosarcoma

MedGen UID:
5178
Concept ID:
C0016057
Neoplastic Process
Synonym: Fibrosarcoma (disease)
SNOMED CT: Fibrosarcoma (443250000); Fibrosarcoma (53654007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0100244
Monarch Initiative: MONDO:0005164
Orphanet: ORPHA2030

Definition

A fibroblastic sarcoma is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrosarcoma
Follow this link to review classifications for Fibrosarcoma in Orphanet.

Conditions with this feature

Diaphyseal medullary stenosis-bone malignancy syndrome
MedGen UID:
350613
Concept ID:
C1862177
Disease or Syndrome
Diaphyseal medullary stenosis with malignant fibrous histiocytoma is an autosomal dominant bone dysplasia characterized by pathologic fractures due to abnormal cortical growth and diaphyseal medullary stenosis. The fractures heal poorly, and there is progressive bowing of the lower extremities. In 2 families, affected individuals also showed a limb-girdle myopathy, with muscle weakness and atrophy. Approximately 35% of affected individuals develop an aggressive form of bone sarcoma consistent with malignant fibrous histiocytoma or osteosarcoma. Thus, the disorder may be considered a tumor predisposition syndrome (summary by Camacho-Vanegas et al., 2012).

Professional guidelines

PubMed

Inarejos Clemente EJ, Diaz Leyva J, Karakas SP, Duarte AM, Mas TR, Restrepo R
Radiographics 2023 Nov;43(11):e230064. doi: 10.1148/rg.230064. PMID: 37883305
Tlemsani C, Larousserie F, De Percin S, Audard V, Hadjadj D, Chen J, Biau D, Anract P, Terris B, Goldwasser F, Pasmant E, Boudou-Rouquette P
Int J Mol Sci 2023 Jan 10;24(2) doi: 10.3390/ijms24021361. PMID: 36674874Free PMC Article
Rashid T, Noyd DH, Iranzad N, Davis JT, Deel MD
Clin Perinatol 2021 Mar;48(1):117-145. doi: 10.1016/j.clp.2020.11.007. PMID: 33583500

Recent clinical studies

Etiology

Wu H, Laskin WB
Surg Pathol Clin 2024 Mar;17(1):83-96. Epub 2023 Aug 8 doi: 10.1016/j.path.2023.07.003. PMID: 38278610
Liu CK, Williamson JE 3rd, Milman T, Lally SE, Shields CL
Ophthalmic Plast Reconstr Surg 2022 Nov-Dec 01;38(6):e180-e183. Epub 2022 Jul 4 doi: 10.1097/IOP.0000000000002236. PMID: 35793663
Brčić I, Rosenberg AE
Semin Diagn Pathol 2021 Nov;38(6):163-169. Epub 2021 May 12 doi: 10.1053/j.semdp.2021.04.005. PMID: 34049746
Bennett G, Brown A, Mudd G, Huxley P, Van Rietschoten K, Pavan S, Chen L, Watcham S, Lahdenranta J, Keen N
Mol Cancer Ther 2020 Jul;19(7):1385-1394. Epub 2020 May 12 doi: 10.1158/1535-7163.MCT-19-1092. PMID: 32398269
Spiguel A
Cancer Treat Res 2014;162:203-23. doi: 10.1007/978-3-319-07323-1_10. PMID: 25070237

Diagnosis

Wu H, Laskin WB
Surg Pathol Clin 2024 Mar;17(1):83-96. Epub 2023 Aug 8 doi: 10.1016/j.path.2023.07.003. PMID: 38278610
Warmke LM, Yu W, Meis JM
Surg Pathol Clin 2024 Mar;17(1):119-139. Epub 2023 Aug 5 doi: 10.1016/j.path.2023.06.009. PMID: 38278601
Folpe AL
Histopathology 2014 Jan;64(1):12-25. Epub 2013 Nov 22 doi: 10.1111/his.12282. PMID: 24266941
Fisher C
Adv Anat Pathol 2004 Jul;11(4):190-201. doi: 10.1097/01.pap.0000131773.16130.aa. PMID: 15220822
Fisher C
Virchows Arch 2004 Sep;445(3):215-23. Epub 2004 Jun 2 doi: 10.1007/s00428-004-1038-9. PMID: 15173943

Therapy

Radaelli S, Stacchiotti S, Casali PG, Gronchi A
Expert Rev Anticancer Ther 2014 Jun;14(6):689-704. Epub 2014 Feb 20 doi: 10.1586/14737140.2014.885840. PMID: 24555529
Wilkins RM, McLeod RA, Reiman HM, Pritchard DJ
Orthopedics 1985 Jan;8(1):141-3. doi: 10.3928/0147-7447-19850101-22. PMID: 4094949
Ducatman BS, Scheithauer BW
Cancer 1983 Mar 15;51(6):1028-33. doi: 10.1002/1097-0142(19830315)51:6<1028::aid-cncr2820510610>3.0.co;2-3. PMID: 6821867
Gerner RE, Moore GE, Pickren JW
Ann Surg 1975 Jun;181(6):803-8. doi: 10.1097/00000658-197506000-00007. PMID: 1138630Free PMC Article
Gane NF, Lindup R, Strickland P, Bennett MH
Br J Cancer 1970 Dec;24(4):705-11. doi: 10.1038/bjc.1970.84. PMID: 5503597Free PMC Article

Prognosis

Haseli S, Mansoori B, Christensen D, Abadi A, Pooyan A, Shomal Zadeh F, Mau B, Khalili N, Murphey M, Chalian M
Radiographics 2023 Aug;43(8):e230005. doi: 10.1148/rg.230005. PMID: 37440448
Davis JL, Rudzinski ER
Surg Pathol Clin 2020 Dec;13(4):739-762. doi: 10.1016/j.path.2020.08.009. PMID: 33183731
Ferrari A, Orbach D, Sultan I, Casanova M, Bisogno G
Semin Fetal Neonatal Med 2012 Aug;17(4):231-238. Epub 2012 May 25 doi: 10.1016/j.siny.2012.05.003. PMID: 22633289
Dorfman HD, Czerniak B
Cancer 1995 Jan 1;75(1 Suppl):203-10. doi: 10.1002/1097-0142(19950101)75:1+<203::aid-cncr2820751308>3.0.co;2-v. PMID: 8000997
Chung EB, Enzinger FM
Cancer 1976 Aug;38(2):729-39. doi: 10.1002/1097-0142(197608)38:2<729::aid-cncr2820380216>3.0.co;2-z. PMID: 974993

Clinical prediction guides

Sun TT, Cheng NH, Cao DY, Peng P
J Ovarian Res 2020 Dec 8;13(1):142. doi: 10.1186/s13048-020-00749-x. PMID: 33292402Free PMC Article
Bennett G, Brown A, Mudd G, Huxley P, Van Rietschoten K, Pavan S, Chen L, Watcham S, Lahdenranta J, Keen N
Mol Cancer Ther 2020 Jul;19(7):1385-1394. Epub 2020 May 12 doi: 10.1158/1535-7163.MCT-19-1092. PMID: 32398269
Kang P, Li PL, Peng JY, Ni M, Jia GJ, Jia W
J Craniofac Surg 2020 May/Jun;31(3):825-828. doi: 10.1097/SCS.0000000000006224. PMID: 32049915
Guo J, Xu B, Han Q, Zhou H, Xia Y, Gong C, Dai X, Li Z, Wu G
Cancer Res Treat 2018 Apr;50(2):445-460. Epub 2017 May 10 doi: 10.4143/crt.2016.572. PMID: 28494534Free PMC Article
Ducatman BS, Scheithauer BW
Cancer 1983 Mar 15;51(6):1028-33. doi: 10.1002/1097-0142(19830315)51:6<1028::aid-cncr2820510610>3.0.co;2-3. PMID: 6821867

Recent systematic reviews

Gu Y, Becker MA, Müller L, Reuss K, Umlauf F, Tang T, Menger MD, Laschke MW
Cells 2023 Jun 22;12(13) doi: 10.3390/cells12131692. PMID: 37443725Free PMC Article
Haider AS, Palmisciano P, Sagoo NS, Bin Alamer O, El Ahmadieh TY, Pan E, Garzon-Muvdi T
Clin Neurol Neurosurg 2022 Mar;214:107127. Epub 2022 Jan 12 doi: 10.1016/j.clineuro.2022.107127. PMID: 35151057
Coca-Pelaz A, Mäkitie AA, Strojan P, Corry J, Eisbruch A, Beitler JJ, Nuyts S, Smee R, Langendijk JA, Mendenhall WM, Piazza C, Rinaldo A, Ferlito A
Adv Ther 2021 Jan;38(1):90-108. Epub 2020 Nov 13 doi: 10.1007/s12325-020-01556-y. PMID: 33184778Free PMC Article
Guerrero-Pérez F, Vidal N, López-Vázquez M, Sánchez-Barrera R, Sánchez-Fernández JJ, Torres-Díaz A, Vilarrasa N, Villabona C
Pituitary 2021 Feb;24(1):117-129. doi: 10.1007/s11102-020-01073-9. PMID: 32785833
Surov A, Gottschling S, Wienke A, Meyer HJ, Spielmann RP, Dralle H
Anticancer Res 2015 Oct;35(10):5185-91. PMID: 26408676

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